title: Endonasal endoscopic transsphenoidal pituitary surgery: early experience and outcome in paediatric Cushing's disease.
abstract: BACKGROUND
  Selective adenomectomy remains the first-line treatment for Cushing's disease (CD), until recently by microscopic transsphenoidal pituitary surgery. Endonasal transsphenoidal endoscopic surgery (ETES) is emerging as a novel, less invasive treatment for pituitary adenomas and has become the optimal surgical approach.
  
  
  OBJECTIVE
  There are no published series for the treatment of paediatric CD by ETES, and we report our centre's preliminary results.
  
  
  METHODS
  Retrospective analysis.
  
  
  METHODS
  Six paediatric patients (median age 15·8 years; range 11·7-17·0 years) fulfilled standard diagnostic criteria for CD. Preoperatively, no abnormality was identified on pituitary MR scanning in 3 (50%) patients, one had a macroadenoma. Bilateral petrosal sinus sampling demonstrated central ACTH secretion (IPS/P ACTH ratio ≥3·0, post-CRH) in 3/6 (50%) patients. The same neurosurgeon and endoscopic nasal surgeon undertook all the operations.
  
  
  METHODS
  Therapeutic outcome and rate of complications.
  
  
  RESULTS
  Clinical recovery and biochemical 'cure' were achieved in 5 (83%) patients, and a corticotroph adenoma was confirmed histologically in all cured cases. One case developed post-operative CSF leak requiring lumbar drain insertion and patching. At a mean interval of 4·7 years (0·1-10·8 years) post-operatively, cured patients have shown no recurrence. One patient, with a large diffuse adenoma requiring more extensive surgery, has panhypopituitarism, and another patient has GH and gonadotrophin deficiencies.
  
  
  CONCLUSIONS
  Our experience shows that ETES for removing corticotroph adenomas in children, in most cases not visualized on MRI, is minimally invasive and gave excellent post-operative recovery/results. In skilled hands, this technique provides an alternative to conventional transsphenoidal microscopic surgery in managing paediatric CD.
authors.affiliation: Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
authors.forename: Helen L
authors.initials: HL
authors.lastname: Storr
authors.forename: William M
authors.initials: WM
authors.lastname: Drake
authors.forename: Jane
authors.initials: J
authors.lastname: Evanson
authors.forename: Matthew
authors.initials: M
authors.lastname: Matson
authors.forename: Dan M
authors.initials: DM
authors.lastname: Berney
authors.forename: Ashley B
authors.initials: AB
authors.lastname: Grossman
authors.forename: Scott A
authors.initials: SA
authors.lastname: Akker
authors.forename: John P
authors.initials: JP
authors.lastname: Monson
authors.forename: Ghassan
authors.initials: G
authors.lastname: Alusi
authors.forename: Martin O
authors.initials: MO
authors.lastname: Savage
authors.forename: Ian
authors.initials: I
authors.lastname: Sabin
mesh_terms: D000236:Adenoma / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D002648:Child; D013507:Endocrine Surgical Procedures / Q000379:methods*; D004724:Endoscopy / Q000379:methods*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009296:Nasal Cavity; D047748:Pituitary ACTH Hypersecretion / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D010902:Pituitary Gland / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D011859:Radiography; D015203:Reproducibility of Results; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000324:Adrenocorticotropic Hormone